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Child's Nervous System : ChNS :... Sep 2023Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors...
PURPOSE
Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed. We present the surgical techniques, pitfalls, and their results.
MATERIAL AND METHODS
Eighty patients ranging in age from 3 months to 21 years old, and treated over 3 decades were reviewed. Hydrocephalus caused the main presenting symptoms and was noted in 74 patients. It was treated prior to the craniotomy for tumor resection with endoscopic third ventriculostomy (ETV) in 33, external ventricular drainage in 26, and precraniotomy shunt in 15. Nine patients had ETV together with endoscopic biopsy. All patients had a parieto-occipital craniotomy in a prone position. Through a tentorial section, a gross total resection of the tumor was attempted except for germinomas.
RESULTS
The tumor pathology showed 32 germ cell tumors (GCT), 22 benign astrocytomas, 13 pineal parenchymal tumors, 5 ATRTs, 3 papillary tumors, and 5 others. Of GCTs, 18 were teratomas. The extent of resection consisted of 55 gross total resections, 13 subtotal resections, 10 partial, and 2 biopsies with one postoperative death. Hemiparesis in 2, cerebellar ataxia in another 2, and hemiballismus in 1 were transient and improved over time. One had permanent hemisensory loss and another patient had bilateral oculomotor palsy. Postoperative homonymous hemianopia occurred in 2 patients but subsided over a short period of time. Parinaud's sign was noted in 24 patients, of which 16 were transient.
CONCLUSION
The posterior interhemispheric OT approach provides a safe route and comfortable access to the pineal region in children. A great majority of postoperative neurological complications are the results of direct manipulations of the midbrain at tumor resection. Identification and preservation of the tumor-brain interface are of paramount importance. GCTs other than teratomas are treated with neoadjuvant chemotherapy and may eliminate the need for craniotomy. Exophytic midbrain JPAs are amenable to resection.
Topics: Child; Humans; Pinealoma; Retrospective Studies; Brain Neoplasms; Pineal Gland; Postoperative Complications; Teratoma
PubMed: 35821434
DOI: 10.1007/s00381-022-05595-4 -
British Journal of Cancer Mar 2021Increased sarcoma and melanoma risks after hereditary retinoblastoma are well established, whereas less is known about epithelial subsequent malignant neoplasms (SMNs)...
BACKGROUND
Increased sarcoma and melanoma risks after hereditary retinoblastoma are well established, whereas less is known about epithelial subsequent malignant neoplasms (SMNs) and risks for multiple (≥2) SMNs.
METHODS
Leveraging long-term follow-up and detailed histologic information, we quantified incident SMN risk among 1128 hereditary and 924 nonhereditary retinoblastoma survivors (diagnosed 1914-2006; follow-up through 2016). Standardised incidence ratios (SIRs) compared cancer risk after retinoblastoma relative to the general population. We estimated cumulative incidence accounting for competing risk of death.
RESULTS
Hereditary survivors had statistically significantly increased SMN risk (N = 239; SIR = 11.9; 95% confidence interval [CI] 10.4-13.5), with SIRs >80-fold for sarcomas, nasal cavity tumours and pineoblastoma. Significantly increased risks were also observed for melanoma and central nervous system, oral cavity and breast SMNs (SIRs = 3.1-17), but not the uterus, kidney, lung, bladder, pancreas or other types. Cumulative incidence 50 years following hereditary retinoblastoma was 33.1% (95% CI 29.0-37.2) for a first SMN and 6.0% (95% CI 3.8-8.2) for a second SMN. SMN risk was not increased after nonhereditary retinoblastoma (N = 25; SIR = 0.8; 95% CI 0.5-1.2).
CONCLUSION
Beyond the established sarcoma and melanoma risks after hereditary retinoblastoma, we demonstrate increased risk for a more limited number of epithelial malignancies than previously suggested. Cumulative incidence estimates emphasise long-term SMN burden after hereditary retinoblastoma.
Topics: Adult; Cancer Survivors; Female; Follow-Up Studies; Genetic Predisposition to Disease; Humans; Incidence; Male; Middle Aged; Neoplasms, Second Primary; Prognosis; Retinal Neoplasms; Retinoblastoma; Survival Rate; United States
PubMed: 33473166
DOI: 10.1038/s41416-020-01248-y -
Arquivos de Neuro-psiquiatria Mar 2008Pineoblastomas are uncommon pineal tumors, which demonstrate rapid growing and poor prognosis. We report the case of a 43-year-old man with an enhancing pineal region...
Pineoblastomas are uncommon pineal tumors, which demonstrate rapid growing and poor prognosis. We report the case of a 43-year-old man with an enhancing pineal region mass, which showed restriction of the diffusion on diffusion-weighted (DW) MR images. The surgical biopsy defined the diagnosis of pineoblastoma and the therapy was initiated with radiation and chemotherapy. Three months later, the follow-up MR imaging showed areas suggestive of necrosis and the DW images demonstrate no significant areas of restricted diffusion. The differential diagnosis of pineal region masses that could show restriction of diffusion is discussed.
Topics: Adult; Brain Neoplasms; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Follow-Up Studies; Humans; Male; Pineal Gland; Pinealoma
PubMed: 18392417
DOI: 10.1590/s0004-282x2008000100015 -
Medicine Jul 2023Tumors of the pineal region (TPRs) are rare neoplasms that are surgically challenging to resect. Conventional treatment strategies are available, but gamma knife...
Tumors of the pineal region (TPRs) are rare neoplasms that are surgically challenging to resect. Conventional treatment strategies are available, but gamma knife radiosurgery (GKRS) is an alternative approach. This study presents a single-center experience with GKRS performed for TPR with and without histopathological diagnoses. The cases of 25 patients with TPRs treated with GKRS were retrospectively analyzed. Thirteen of these 25 patients had histopathological confirmation, and 13 had elevated serum alpha-fetoprotein and beta-human chorionic gonadotropin levels. The 25 patients had a mean follow-up duration of 61 months. The total response rate to GKRS was 60%, and a 53.8% decrease in the alpha-fetoprotein and beta human chorionic gonadotropin levels was observed. The findings of this study indicate that GKRS is a safe procedure for TPRs, even in the event of insufficient histopathological findings. This treatment approach provides increased Karnofsky performance scores and an extended life expectancy.
Topics: Humans; Follow-Up Studies; Radiosurgery; alpha-Fetoproteins; Pinealoma; Retrospective Studies; Pineal Gland; Brain Neoplasms; Chorionic Gonadotropin; Treatment Outcome
PubMed: 37417623
DOI: 10.1097/MD.0000000000034005 -
Cancer Reports (Hoboken, N.J.) Sep 2022Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes...
BACKGROUND
Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes neoadjuvant chemotherapy (NC) followed by radiotherapy (RT) or RT at a higher dose and larger field. These recommendations are based on studies focused mostly on children; it is currently unclear whether this treatment is applicable to adults.
CASE
We present a case of a 23-year-old adult male with no underlying pathologies, drug allergies, or family history of cancer, who presented for medical evaluation with blurred vision, diplopia, forgetfulness, and weight loss starting 3-4 months before the evaluation. Clinical examination indicated Parinaud's Syndrome. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a pineal tumor with ependymal dissemination in both lateral ventricles, which was causing obstructive hydrocephalus. The patient had surgery consisting of ventriculostomy, Holter shunt insertion, cisternal ventricular intubation, and cisterna magna anastomosis to improve ventricular drainage. Pathology confirmed pineal germinoma. Cerebrospinal fluid cytology and MRI of the axis were negative. Four cycles of NC were given to the patient (carboplatin, etoposide, and ifosfamide), with reduced dosage. Once a partial volumetric response was confirmed, whole-ventricular radiotherapy (WVR) was initiated with a total tumor bed dose of 45 Gy over 25 sessions in 5 weeks. Optimum clinical results were observed, and no short-term (<90 day) radiation toxicity was observed. The patient was able to resume his normal activities soon after treatment. Follow-ups over 2 years post-surgery indicated continued control of the lesion and absence of symptoms except for mild diplopia.
CONCLUSION
Although this is a case report, these data suggest that a reduced NC course and WVR may effectively treat adult GN. This protocol likely decreases the risk of undesirable NC and RT secondary effects, while providing excellent local control; however, using a narrower RT field is not recommended.
Topics: Adult; Brain Neoplasms; Child; Diplopia; Germinoma; Humans; Male; Pineal Gland; Pinealoma; Young Adult
PubMed: 35347869
DOI: 10.1002/cnr2.1611 -
AJNR. American Journal of Neuroradiology Sep 2010Although pineoblastoma is the main brain abnormality associated with hereditary retinoblastoma, recent studies suggest an association with pineal cysts. This association...
BACKGROUND AND PURPOSE
Although pineoblastoma is the main brain abnormality associated with hereditary retinoblastoma, recent studies suggest an association with pineal cysts. This association is important because some pineoblastomas mimic pineal cysts. If there is a relationship, then radiologists should be aware of it because diagnostic confusion is possible. Mental retardation and congenital brain anomalies are also reported in patients with retinoblastoma, mostly in combination with 13q deletion syndrome. In this retrospective study, the presence of brain abnormalities on MR images in a large group of consecutive patients with retinoblastoma is evaluated.
MATERIALS AND METHODS
Brain MR images of 168 patients with retinoblastoma from 1989 to 2009 were evaluated by 2 radiologists for tumors, structural anomalies, myelinization, and coincidental findings. Clinical records were reviewed for laterality, heredity, and the presence of the 13q deletion syndrome.
RESULTS
The hereditary group (patients with bilateral and unilateral proved RB1-germline mutation) included 90 (54%) of 168 patients. Seven patients had 13q deletion syndrome. Normal findings on brain MR images were seen in 150 (89%) patients. Five pineoblastomas were detected, all in patients with hereditary retinoblastoma (5.5% in the hereditary subgroup). Nine pineal cysts were detected (2.2% in the hereditary subgroup). Corpus callosum agenesis was found in 1 patient and a Dandy-Walker variant in 1 patient, both in combination with 13q deletion syndrome.
CONCLUSIONS
Pineoblastoma is associated with hereditary retinoblastoma, and structural brain abnormalities are restricted to patients with the 13q deletion syndrome. The incidence of pineal cysts in patients with retinoblastomas is similar to that in healthy children and is not associated with hereditary retinoblastoma.
Topics: Aicardi Syndrome; Brain; Brain Neoplasms; Child, Preschool; Chromosome Deletion; Chromosome Disorders; Chromosomes, Human, Pair 13; Dandy-Walker Syndrome; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Pineal Gland; Pinealoma; Retinal Neoplasms; Retinoblastoma
PubMed: 20413604
DOI: 10.3174/ajnr.A2102 -
Journal of Medical Case Reports Jun 2022We report a rare case in medical literature of a patient with pineal gland teratoma and uncommon metastases. Usually, metastases of this kind of tumor are located in...
BACKGROUND
We report a rare case in medical literature of a patient with pineal gland teratoma and uncommon metastases. Usually, metastases of this kind of tumor are located in several organs such as lung and breast, but here we found metastases to the spinal cord and vertebrae.
CASE PRESENTATION
A 35-year-old Asian white man presented with diplopia and acute neural symptoms in the lower limbs such as numbness, tingling, and paralysis. His medical history was notable for pineal teratoma, treated 1 year previously with surgery, radiotherapy, and chemotherapy. Physical examination of the lower limbs showed absent reflexes and sensation with muscle power scale score of 1 in both limbs. Magnetic resonance imaging of brain and spine revealed many lesions in various locations, most compatible with neural, spinal, and vertebral metastases. Unfortunately, the patient died suddenly before any intervention was carried out.
CONCLUSION
It is extremely rare for pineal region teratoma to metastasize to the spinal cord and vertebrae, thus more vigilant observation and examination should be provided to patients with pineal teratoma to detect any new lesions and prevent them from becoming dangerous.
Topics: Adult; Brain Neoplasms; Humans; Magnetic Resonance Imaging; Male; Pinealoma; Teratoma
PubMed: 35739572
DOI: 10.1186/s13256-022-03454-z -
Neuroradiology Jul 2016Pineal cysts are a common incidental finding on brain MRI with resulting difficulties in differentiation between normal glands and pineal pathologies. The aim of this... (Comparative Study)
Comparative Study
INTRODUCTION
Pineal cysts are a common incidental finding on brain MRI with resulting difficulties in differentiation between normal glands and pineal pathologies. The aim of this study was to assess the size and morphology of the cystic pineal gland in children (0-5 years) and compare the findings with published pineoblastoma cases.
METHODS
In this retrospective multicenter study, 257 MR examinations (232 children, 0-5 years) were evaluated regarding pineal gland size (width, height, planimetric area, maximal cyst(s) size) and morphology. We performed linear regression analysis with 99 % prediction intervals of gland size versus age for the size parameters. Results were compared with a recent meta-analysis of pineoblastoma by de Jong et al.
RESULTS
Follow-up was available in 25 children showing stable cystic findings in 48 %, cyst size increase in 36 %, and decrease in 16 %. Linear regression analysis gave 99 % upper prediction bounds of 10.8 mm, 10.9 mm, 7.7 mm and 66.9 mm(2), respectively, for cyst size, width, height, and area. The slopes (size increase per month) of each parameter were 0.030, 0.046, 0.021, and 0.25, respectively. Most of the pineoblastomas showed a size larger than the 99 % upper prediction margin, but with considerable overlap between the groups.
CONCLUSION
We presented age-adapted normal values for size and morphology of the cystic pineal gland in children aged 0 to 5 years. Analysis of size is helpful in discriminating normal glands from cystic pineal pathologies such as pineoblastoma. We also presented guidelines for the approach of a solid or cystic pineal gland in hereditary retinoblastoma patients.
Topics: Brain Neoplasms; Central Nervous System Cysts; Child, Preschool; Diagnosis, Differential; Europe; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Pineal Gland; Pinealoma; Reference Values; Reproducibility of Results; Retrospective Studies; Sensitivity and Specificity
PubMed: 27130617
DOI: 10.1007/s00234-016-1683-0 -
Journal of Radiosurgery and SBRT 2018Stereotactic radiosurgery (SRS) is a treatment option, which is capable of pinpoint irradiation and thus, reduce the potential late complications. However, any type of...
Stereotactic radiosurgery (SRS) is a treatment option, which is capable of pinpoint irradiation and thus, reduce the potential late complications. However, any type of radiation therapy is not recommended for brain tumor patients under the age of 3 years. SRS is not routinely recommended for patients than 2 years of age in consideration of infant skull brittleness for frame fixation, and lack of treatment evidence for the safety and effects of stereotactic radiosurgery in infants. We experienced the case of Gamma Knife treatment upon an infantile pineoblastoma where repeated tumor excision had already been perfromed and chemotherapy resistance was apparent. Radiosurgery resulted in symptom improvement and dramatic tumor shrinkage on MRI after radiosurgery. Therefore, here we report on the difficulty and usefulness of Gammaknife radiosurgery in this infant patient.
PubMed: 29988319
DOI: No ID Found -
Chinese Neurosurgical Journal May 2022To analyze clinically and radiologically the surgical outcome like residual disease, progression of disease, recurrence, disabilities, event-free survival (EFS), and...
BACKGROUND
To analyze clinically and radiologically the surgical outcome like residual disease, progression of disease, recurrence, disabilities, event-free survival (EFS), and mortality of different infra-tentorial tumor subtypes in children and adults of a strictly non-migratory and ethnic population.
METHODS
The 410 histologically proved, out of 589, infra-tentorial brain tumor patients were analyzed clinically and by the imaging post-surgically in a single tertiary center for an ethnic region. In this analytico-observational study, retrospectively postoperative records of 589 infra-tentorial brain tumors from November 1998 to December 2018 (20 years) were retrieved, scrutinized, and compiled. The post-operative clinic-radiological records of 410 patients with proved histopathological examination results were included. Statistical law of variance was applied where-ever necessary.
RESULTS
The 63.2% of the all 410 operated infra-tentorial brain tumors were males while females predominated in meningiomas and pineoblastomas. About 31.7% infra-tentorial tumors were children (below 18 years). About 54.1% cases were histologically malignant. The residual tumors comprised 40.2% and symptoms of disease-progression occurred in 10.9%. The tumor recurrence occurred in 14.3% while 6.0% patients developed severe disability. The overall mortality was 11.4% but 18.9% in malignant tumors. The event-free survival (EFS) for all the patients was 66.0%, patients with malignancies had 47.7% and benign group had 87.7%.
CONCLUSION
The study, surgical outcome of infra-tentorial brain tumor subtypes in children and adults (approx. 1/3rd of patients being children), conducted in a tertiary center at a remote land-locked location with non-migratory ethnic population as its catchment area, has a significant epidemiological value for the community and the region.
PubMed: 35505445
DOI: 10.1186/s41016-022-00275-3